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Original Article

Goldenhar syndrome: craniofacial treatment protocol

2013, Volumen 43, Número 1
Estela Luque Martín, Maria Cavaller Fortuny, Joana Navarro Lara, M. Lluïsa Tobella Camps, Alejandro Rivera Baró
Máster de Ortodoncia. Hospital Sant Joan de Déu. Barcelona

Introduction. The Goldenhar syndrome is a polymalformative pattern characterized by hemifacial microsomia, as well as auricular, ocular and vertebral anomalies. Between 7-25% of the cases are associated to lip and palate cleft. Objectives. Analysing clinical manifestations for 36 patients who have undergone treatment at the Hospital Universitario Sant Joan de Déu. Describing the maxilofacial treatment for 13 pediatric patients seen in the craneofacial unit. Establishing a therapeutical protocol for patients with this syndrome. Materials and methods. A retrospective study was carried out with a sample of 36 patients who manifested the Goldenhar syndrome and who have been born in Hospital Sant Joan de Déu between years 1976-2011. Their clinical characteristics and the craneofacial treatment received were analysed. They were distributed according to how severe their case was and following the Pruzansky and Kaban classification into type I, type IIa, IIb and III. Results. It was observed that types III and IIB, which are the most severe cases, required a previous phase for the orthodontic treatment to be carried out. It consisted on a bone graft and/or distraction osteogenesis. Nonetheless, types I and IIa, the least severe cases, underwent a surgical treatment from the start. According to the phase of the children’s development and the severity of the syndrome, the following phases of the treatment have been established: neonatal period (0-2 y.o): facial surgery of soft tissues and early maxillary orthopaedics; mixed dentition (7-12 y.o): correction/stimulation of the bone growth (functional appliance, bone graft and/or distraction), and permanent dentition (over 13 y.o): surgical orthodontic treatment. Conclusion. No medical literature has been found regarding the minimal diagnostical criteria, which hinders and postpones the detection and the correct diagnosis for the syndrome. The treatment for malformations associated to the Goldenhar syndrome is planified using a protocol in accordance to the severity of the clinical pattern.  (Rev Esp Ortod. 2013;43(1):040-047)

Key words:
Goldenhar syndrome. Craneofacial treatment. Osteogenic distraction.
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